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Tumores cardiacos

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Clase acerca de Tumores Cardiacos.

Clase acerca de Tumores Cardiacos.

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    Tumores cardiacos Tumores cardiacos Presentation Transcript

    • Tumores Cardiacos Abraham Villalobos F RMi
    • Introducción • Prevalencia en serie de autopsias <0.1%. • Las metástasis a corazón son 20 veces más frecuentes. • Suelen ser asintomáticos. • Los síntomas comúnmente se confunden con las patologías cardiacas más frecuentes. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996; 77:107.
    • Med Assoc J 1934;30:368-373. TABLE I Frequency of Primary Heart Tumors N in Autopsy TU 5. Shelbume tures which Series % SA. Primary tumors led to a logical and 1935;9334rk349. 6. Pollia JA, Cog01 W. Some 1936;27:32%333. 8,550 4 0.047 Lymburner4 7. Scott RW, Garvin CF. Tumors 1,200 3 0.25 Shelburnej 17~43 l-436. Pollia and Gogo 12,000 0 0 8. Benjamin HG. Primary fibmm Scott and Garvin’ 11,100 0 0 9. Ravid JM, Sachs 1. Tumors Benjamin* 40,000 12 0.03 “sarcoma of the left auricle and t of the mesentety and alimentary Ravid and Sachs9 1,888 0.053 10. Straus R, Merliss R. Primary 480,33 1 i3 0.0017 AMA survey 11938-l 942)‘O 11. Leach WB. Primary neoplasm 1,550 3 0.19 Straus and Merliss’O 12. Whotton CM. primary malig Leach’ 1 6,275 0.016 cer lY49;2:245-260. WhortonJ2 20,337 ; 0.015 13. Amsterdam HJ, Grayzel DM Amsterdam et alI3 5,000 I 0.02 heart. Am Heart J 1949;37:291-300. Prichard14 4,200 4 0.095 14. Richard RW. Tumors of t Saphir’5 7,889 2 0.025 hundred and fifty cases. Arch P 15. Saphir 0. ticoplasms of the 18,328 23 0.125 FineI ogy of the Heart. Springfield, IL 3,914 1 0.026 Heath” 16. Fine G. Primary tumors of Baumann and Clavadetscher’* 2,070 4 0.19 JE. eds. Cardiovasc Clin 5. Clini Wold and Lie’9 23,673 41 0.17 Davis 1973:207-238. 10,261 2: 0.049 Fabian and Rose20 17. Heath D. Pathology of cardia 26,980 0.078 Schanz and Schneide?’ 18. Baumann RP. Clavadetsfher Schwesinger et aI22 25,855 9 0.035 nanntes myxom, mit embolie in tibrosarkom. Schwviz med Wschr Guang-yingz3 7,423 4 0.054 19. Wold LE. Lie JT. Cardiac m 12,485 7 0.056 Lam et al24 1Y8~101:219-233. Total 73 1,309 157 0.02 1 20. Fabian JT. Rose AG. Tumou 1982;61:71-77. Percentage is based on the total number of autopsies performed (N) and 21. Schanz II, Schneider I. Endo the number of detected tumors (TU). Schwriz med Wschr 19X4;114:850-8 22. Schwesinger G, Meyer B, v moren. Z ges inn Med 19pA;3Y:36 23. Guang-ying L. lncidcnce an From the De FrequencyInternal Medic tumors of the heart. Am J Cardiol 1996; 77:107. II, University of ErlongenReynen K. rtment of of primaryine review of the literature. Thorac Niirnberg, t? Ache Stadtmauerstrasse 29, 9 10.54 Erlangen, Ger24. lam KY, Dickens P, Lam
    • Manifestaciones Clínicas • Determinadas por la localización del tumor. • Los mecanismos son variados: • • • Obstrucción de la circulación -> Falla cardiaca. Interferencia con las válvulas -> regurgitación. Invasión directa del miocardio -> alteración de la contractilidad, arritmias, bloqueos, derrame pericárdico con/sin tamponade. Salcedo EE, Cohen GI, White RD, Davison MB. Cardiac tumors: diagnosis and management. Curr Probl Cardiol 1992; 17:73.
    • Manifestaciones Clínicas • Invasión al pulmón -> Síntomas pulmonares. ! • Embolización -> Sistémica, la más frecuente. ! • Síntomas constitucionales o sistémicos. Salcedo EE, Cohen GI, White RD, Davison MB. Cardiac tumors: diagnosis and management. Curr Probl Cardiol 1992; 17:73.
    • Evaluación Diagnóstica • Ecocardiografía. • TC y RM. • PET. • Coronariografía. • Biopsia transvenosa. Salcedo EE, Cohen GI, White RD, Davison MB. Cardiac tumors: diagnosis and management. Curr Probl Cardiol 1992; 17:73.
    • Tumores Benignos • 75% de los tumores cardiacos. • Mixomas • • Los más comunes. • El mesénquima de origen puede diferenciarse a tejido neural y/o endotelial. • Productores de VEGF. Histológicamente compuestos de células dispersas en el estroma mucopolisacárido. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682.
    • Tumores Benignos • Mixoma Macroscópicamente RG ■ Volume 20 • Number 4 • • Pedunculados y de consistencia gelatinosa. • • Varía en tamaño que va de 1 a 15 cm de diámetro. La superficie puede ser suave (>70%), vellosa o friable (35%). Peso de 15 a 180 gr. RG Volume 20 • • Number RG ■ ■ Volume 20 Number 4 4 Grebenc et al Grebenc et al 10771077 2. 3. Figures 2–4. Ca (2) Photograph de mass with a varieg atrial myxoma dem latinous mass. A p row) was excised e graph of a left atria gated, focally hem surface. range in size fro Cut sections of t appearance, occ (1,20). 2. 2. 3. 3. Radiologic Fea Clifford-Roberts, W. Primary and secondary neoplasms of 4. heart. Am J Cardiol. Volume 80, 1997: 671-682. the Figures 2–4. Cardiac myxoma, pathologic features. Figures 2–4. Cardiac myxoma, pathologic features. tures of cardiac
    • Tumores Benignos Mixoma • Manifestaciones Clínicas • • • 80% se originan en la AI, el resto de la AD. • Pueden simular enfermedades del tejido conectivo. (34%). 67% presentan datos de estenosis valvular mitral. 29% presentan embolización (20% síntomas neurológicos, mayor en hombres). Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. 2001; 80:159.
    • Tumores Benignos • Mixoma Síndrome de Gorlin: • • • • • Múltiples nevos con Ca basocelular secundario. Quistes, fibrosarcomas de la mandíbula. Anomalías esqueléticas. Milia, quistes epidermoides, comedones. Asociado a meduloblastoma, meningioma, fibroma/ fibrosarcoma ovárico, fibroma cardiaco, rabdomioma fetal y quistes linfáticos mesentéricos. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. 2001; 80:159.
    • Tumores Benignos • Complejo de Carney • • • • Mixoma Autosómico dominante. Múltiples tumores: mixomas intra y extracardiacos. Schwannomas y varios tumores endócrinos. Anormalidades en la pigmentación: lentiginosis, nevos azules. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. 2001; 80:159.
    • Tumores Benignos Mixoma 5. 6a.
    • RG ■ Volume 20 • Number 4 Tumores Benignos Mixoma 6a.
    • RG ■ Volume 20 • Number 4 Grebenc et al 1081 a. b. Figure 8. Left atrial myxoma in a 61-year-old woman with progressively worsening dyspnea. (a) Non-contrast-enhanced chest CT scan (mediastinal window) shows an ovoid left atrial mass that is hypoattenuating with respect to the surrounding blood. (b) Contrast-enhanced chest CT scan (mediastinal window) shows heterogeneous enhancement of the myxoma, which is attached to the interatrial septum. The contrast material–opacified blood outlines the margins of the lobular mass. Note the large bilateral pleural effusions and bibasilar atelectasis. atrioventricular valve during diastole may also be demonstrated. Doppler echocardiography can be used to evaluate associated valvular regurgitation or stenosis (5,19,31). Contrast material–enhanced chest CT usually demonstrates a well-defined spherical or ovoid intracavitary mass, typically with lobular (but occasionally smooth) contours. Tumor attenuation is lower than that of unopacified blood. Intravenous administration of contrast material helps to better define the lesion as a mass of low attenuation surrounded by the enhancing intracardiac blood (Fig 8). Heterogeneity is a common feature of myxoma and is thought to reflect hemorrhage, necrosis, cyst formation, fibrosis, calcification, or ossification (Fig 9) (32). Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No 4:2000:1073-1103. a. b.
    • Tumores Benignos • Tratamiento y Pronóstico • • Cirugía: mortalidad <5% a 6 años. • Mixoma Recurrencia: 2-5% Complicaciones: arritmias u otras anomalías en la conducción (26%). Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. 2001; 80:159.
    • Tumores Benignos 1082 July-August 2000 • Fibroelastoma papilar. • • • • El segundo en frecuencia. 55% hombres. 60% > años. 2 a 70 mm. Media 9 mm. a. Figure 13. Papillary fibroelastoma in a 69-year-old man with chronic atrial fibrillation. (a) Transthoracic four-chamber echocardiogram shows a rounded echogenic mass (arrow) attached to the apex of the left ventricle (LV). LA = left atrium, RA = right atrium, LV = left ventricle. (b) Axial, cine GRE (66/5) MR image demonstrates a 1-cm mass at the apex of the left ventricle (arrowhead). (c) Photograph of the excised specimen shows a gelatinous, multilobular, papillary mass. 80% en válvulas (aórtica>mitral>tricúpide>pulmonar). Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: Microscopic Features.—Papillary fibroelastomas are avascular papillomas lined by a single 671-682. b.
    • Tumores Benignos • • • • Clínica: embolización: EVC, AIT. • Fibroelastoma Papilar Angina, IAM, IC, síncope, síntomas pulmonares, muerte súbita. 30% asintomáticos. Apariencia de anémona de mar. Tratamiento • • Expectante. Cirugía: ≥1 cm, embolización, afectación valvular. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682; Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No 4:2000:1073-1103.
    • RG ■ Volume 20 • Number 4 Tumores Benignos RG ■ Volume 20 • Number 4 Grebenc et al 1083 Rabdomioma • • • Casi exclusivamente en niños <1 año. 80 - 90% relacionados con esclerosis tuberosa. a. Figure 15. Rhabdomyoma in a 3-month-old boy with tach shows diffuse heterogeneous nodular thickening of the left ve rows). (b) Photograph of the cut autopsy specimen of the he throughout the left ventricular myocardium (arrows). b. a. Figure 15. Rhabdomyoma in a 3-month-old boy with tachycardia. (a) Coronal T1-weighted (370/25) MR image shows diffuse heterogeneous nodular thickening of the left ventricular myocardium and interventricular septum (arrows). (b) Photograph of the cut autopsy specimen of the heart shows multiple, firm, white nodules distributed throughout the left ventricular myocardium (arrows). Localizados en paredes ventriculares o válvulas. • La mayoría presentan regresión espontánea. • Cirugía requerida en caso de síntomas. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J 671-682. screening. Affected children may be detected in utero because of nonimmune fetal hydrops and fetal death. Other presenting features include tachyarrhythmias, murmurs, and heart failure. Because the majority of cardiac rhabdomyomas regress spontaneously, surgery is not routinely required. However, patients with life-threatening symptoms, usually those secondary to left ventricular outflow tract obstruction or refractory arrhythmias, respond well to surgical excision (47,49–52). Microscopic Features.—Rhabdomyomas are composed of enlarged, vacuolated cells with Figure 14. Rhabdomyoma, microscopic features. sparse cytoplasm that resemble altered myocytes. High-power photomicrograph (original magnification, Cells stain stain) demonstrates typical vacuolated strongly with periodic-acid Cardiol. Volume 80, 1997: Schiff cells 300; H-E stains due to theirCytoplasmic streaming is a“Spider of rhabdomyoma. high glycogen content. typical cells” are typical and are characterized by a cenartifact that results in “spider cells” (arrows).
    • Tumores Benignos Fibroma also endocrine neoplasms. Both the NAME (nevi, atrial myxoma, neurofibromata, and ephelides) and the LAMB (lentigines, atrial myxoma, and balloon nevi) syndromes are associated with the familial variety of cardiac myxoma. Chromosomal abnormalities for atrial myxoma on chromosome 2 (Carney’s) and chromosome 12 (Kiras-genes) have been described. Myxomas probably present the most varied clinical picture of all primary cardiac neoplasms.6,8,9 Several major syndromes have been observed including presentation with signs of emboli, obstruction to blood flow, and various constitutional syndromes. Fragments of tumor located in the right side of the heart may embolize to the lungs and those in the left side of the heart, of course, to various systemic organs. Diagnosis may be made on occasion by finding typical myxomatous endothelial-like cells which are elongated and spindle shaped with round or oval nuclei and prominent nucleoli in operatively removed emboli. Obstruction to blood flow may occur at the orifice of any valve, most commonly, of course, the mitral valve. Interference with flow through the mitral orifice may mimic signs of mitral stenosis, including signs of pulmonary congestion, diastolic apical rumble, opening snap, and accentuated first heart sounds. A murmur of mitral regurgitation may also be present as a result of chronic damage to the valve leaflets or to interference with proper closure of the valve by tumor. Differentiation between left atrial tumor and primary mitral stenosis is suggested by the influence of position on symptoms and on the intensity of the precordial murmurs and the opening snap. The constitutional symptoms associated with atrial myxomas are protean and include fever, weight loss, Raynaud’s phenomenon, digital clubbing, anemia, elevated erythrocyte sedimentation rate, elevated leuko- • • RG ■ Volume 20 • Number 4 FIGURE 4. Diagram of an intramyocardial fibroma seen at necropsy in a 5-month-old boy (A-07-92) who was found dead in his crib. The neoplasm had been diagnosed by biopsy during the child’s first few days of life. It was considered nonresectable. mately 10% of all patients with myxomas and the transmission is an autosomal dominant one. The familial myxomas occur in younger patients (mean age 25) than the nonfamilial ones and they have less female gender predominance. The myxomas in the familial syndrome are much more liable (50% of the time) to be multiple and to have a ventricular cavity location (13% as compared with about 2% in the nonfamilial or sporadic patients with myxomas). These patients typically have exterior facial freckling; they have noncardiac myxomas (breast or skin) and Raros. El segundo en frecuencia en niños. Músculo ventricular (Izquierdo>Derecho). • Clínica: disfunción miocárdica, falla cardiaca. • • Cirugía: pacientes sintomáticos. Trasplante. Clifford-Roberts, W. Primary features. HighFigure 16. Fibroma, microscopic and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682; Gerenc et al. Primary cardiac and pericardial power photomicrograph (original magnification, 150; neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No 4:2000:1073-1103. H-E stain) demonstrates dense collagen bundles in a
    • F re te g a ( e Tumores Benignos Teratoma • • • a. b. Figure 22. Cardiac paraganglioma in a 13-year-old boy with a heart murmur and hypertension. (a) Axial T1weighted (400/20) MR image demonstrates a round, lobular mass of intermediate signal intensity arising from the interatrial septum and protruding into both atria. (b) Axial proton density–weighted (2,000/20) MR image shows an increase in tumor signal intensity. Intraoperative palpation of this paraganglioma resulted in immediate, severe hypertension and tachycardia. Se originan dentro del pericardio. Therapy and Prognosis.—Surgical Consecuencias mecánicasexcision of cardiac paragangliomas is usually successful and provides complete symptomatic relief (73,75). importantes. particular to cardiac paraganglioSurgical risks mas include hypertensive crisis from intraoperative manipulation of the tumor and fatal hemorrhage due to the high vascularity of these lesions. Cardiopulmonary bypass isolates the heart from the systemic circulation and allows safe manipulation and dissection of the tumor. Because paragangliomas may be infiltrative lesions, extensive resection of the atrial wall may be required for complete excision (80,81). Tratamiento: cirugía in utero o Cesárea y cirugía inmediata programadas. c. Microscopic Features.—Microscopically, cardiac teratomas are similar to benign extrapericardial teratomas. Typically, they contain derivatives of all three germ layers, with mature endodermal, mesodermal, and ectodermal elements (Fig 23) (1). Tissues discovered in intrapericardial teratomas are diverse and include neuroglia, cartilage, skeletal muscle, liver, intestine, pancreas, and glandular tissue. Pathologic Features.—Pericardial teratomas are usually right-sided masses, which typically connect to one of the great vessels via a pedicle. Clinical secondary neoplasms of teratoma is a Clifford-Roberts, W. Primary and Features.—Pericardial the heart. Am J Cardiol. Figure 23. 1997: 671-682; microscopic features. Volume 80, Mature Most teratomas of theteratoma, within the periheart 20, benign germ cell neoplasm that typically - Pathologic correlation. AFIP Archives. Vollie No Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologicaffects Low-power photomicrograph (original magnification, cardial sac; rarely, they can be intramyocardial. infants and children, who present with respira4:2000:1073-1103. 75; Masson trichrome stain) demonstrates neuroretinal Teratoma m f s T w R f c p w a m t c c i a e M o m t m
    • Tumores Benignos Hamartoma/células de Purkinje • • • • • • Pequeños. Células aplanadas. Usualmente en VI, superficies endocárdicas o epicárdicas. Indetectables con técnicas radiológicas. Niños. Taquicardia ventricular incesante. EKG: BRDHH. Estudios electrofisiológicos. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682; Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No 4:2000:1073-1103.
    • Tumores Benignos • • Adipocitos. Lipoma >50% surgen en la región subendocárdica, el resto en válvulas. • b. a. b. Figure 20. Intracardiac lipoma in a 45-year-old woman with palpitations. (a, b) Axial (a) and coronal (b) proton density–weighted (1,091/20) MR images demonstrate a smooth, round, intracavitary right atrial mass with a signal intensity characteristic of fat. (c) Photograph of the specimen demonstrates a well-circumscribed, spherical, yellow mass that was excised from the right atrium. Tamaño pequeño aunque pueden llegar a pesar hasta 4.8 kg. • Clínica: arritmias, anomalías en la conducción. • Diagnóstico por ecocardiografía o RM. • Tto. Cirugía. Radiologic Features.—The most frequent radiographic abnormality in patients with cardiac lipoma is cardiomegaly (68,70,71). A globularshaped heart has also been described (69). Echocardiography typically demonstrates an echogenic, nonmobile mass (68,70). CT and MR imaging are useful for making a tissue-specific diagnosis based on the findings of fat attenuation (Hounsfield measurement 50) and signal intensity c. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682; c. characteristics of fat (Fig 20). Lipomas appear as Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No predominantly homogeneous masses on CT 4:2000:1073-1103. scans but may display internal soft-tissue septa or
    • Tumores Benignos Potencialmente Malignos • Paranglioma • • Tumores neuroendócrinos. Hormonalmente activos o inactivos. Clínica • • • • Inactivos: compresión y tamponade. Activos: producen NE: cefalea, diaforesis, taquicardia, HAS. Usualmente en pericardio. Secuestran el riego sanguíneo del corazón y son difíciles de remover. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682; Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No 4:2000:1073-1103.
    • Tumores Benignos Potencialmente Malignos RG ■ Volume 20 • Number 4 • Paranglioma 1090 July-August 2000 1090 July-August 2000 RG Volume 20 • • Number RG ■■ Volume 20 Number 4 4 Figure 21. Paraganglioma, microscopic features. High-power photomicrograph (original magnification, 300; H-E stain) demonstrates the classic nesting (zellballen) appearance of the paraganglial cells. which are normally predominantly located within the atria. Most lesions have been reported in adult patients with an age range of 18–85 years (mean age, 40 years) (73). The majority of reported cardiac paragangliomas have been catecholamine-producing tumors, and affected patients present with arterial hypertension, headache, palpitations and flushing, (symptoms typical of pheochromocytoma) (73,74). The biochemical laboratory abnormalities that lead to the diagnoa. b. sis of a paraganglioma include elevated levels of a. b. Figure 22. Cardiac paraganglioma in a 13-year-old boy with heart murmur andAm J Cardiol. Axial T1- urinary norepinephrine, vanillylmandelic acid, and Clifford-Roberts, paraganglioma in 13-year-old neoplasms of the heart. hypertension. (a) Axial T1Figure 22. Cardiac W. Primary andasecondaryboy with aaheart murmur and hypertension. (a) Volume 80, 1997: 671-682; weighted (400/20) MR image demonstrates round, lobular mass ofRadiologic -signal intensitycorrelation. the Gerenc et al. Primary cardiac and pericardial neoplasm: intermediate Pathologic arising from AFIP Archives. Vol elevated weighted (400/20) MR image demonstrates aaround, lobular mass of intermediate signal intensity arising from the total metanephrine or20, No levels of plasma interatrial septum and protruding into both atria. (b) Axial proton density–weighted (2,000/20) MR image shows norepinephrine and epinephrine (74). Up to 20% 4:2000:1073-1103. interatrial septum and protruding into both atria. (b) Axial proton density–weighted (2,000/20) MR image shows anan increase in tumor signal intensity. Intraoperative palpation of this paraganglioma resulted in immediate, severe hy-of patients with cardiac paragangliomas have as-
    • Tumores Benignos Potencialmente Malignos Mesotelioma RG ■ Volume 20 • Number 4 Grebenc et al 1097 • • • Grebenc et al 1097 when examined w histochemical tech tures include necr morphism (1). Cy dial fluid is freque the difficulties in d sothelioma cells fr Pathologic Featu mesotheliomas typ pericardial masses space and constric masses are firm, w though there may epicardium, signif rare (Fig 32) (1). Figure 31. Mesothelioma, microscopic features. High-power photomicrograph (original magnification, 400; H-E stain) demonstrates tubulopapillary structures (arrow) characteristic of epithelial mesothelioma. Origen: pleura o pericardio. Constricción. survivals of 6 months to 1 year after diagnosis (1,107). Radiologic Featu patients with peric demonstrates card pericardial effusion or diffuse mediast Chest CT demons dial thickening and (107,108). MR im strates cardiac enc cardial mass, as w effusion (109). Cualquier técnica de imagen y/o pericardiocentesis. Microscopic Features.—Malignant mesothelioma is typically a biphasic tumor composed of epithelial areas, which resemble a carcinoma, and a. b. spindled areas similar to a sarcoma. The epitheFigure 32. Pericardial mesothelioma in a 36-year-old woman with fatigue, malaise, and dyspnea. (a) Axial conlial areas form tubulopapillary structures (Fig 31). trast-enhanced chest CT scan (mediastinal window) demonstrates a lobular, heterogeneous left pericardial mass The spindled areas usually demonstrate some with a large pericardial and bilateral pleural effusions. Note the large areas of low attenuationevidence oflikely repre(arrow), mesotheliomatous differentiation • Raro: origen en nodo AV con bloqueo. when examined with ultrastructural or immuno- senting necrosis, within the tumor. (b) Photograph of the cut specimen of the heart obtained at autopsy shows a diffuse nodular pericardial mass that encases the heart. Scale is in centimeters. histochemical techniques. Other microscopic features include necrosis and extensive cellular pleomorphism (1). Cytologic examination of pericardial fluid is frequently nondiagnostic because of the difficulties in differentiating malignant mesothelioma cells from reactive cells (107,108). b. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682; r-old woman with fatigue, malaise, and dyspnea. (a) Axial conGerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No Pathologic Features.—Malignant pericardial w) demonstrates a lobular, heterogeneous left pericardial mass 4:2000:1073-1103. mesotheliomas typically form multiple coalescing ns. Note the large areas of low attenuation (arrow), likely repre- • Tratamiento: resección + Qx y/o Rx.
    • Tumores Malignos • Constituyen el 15% de los tumores cardiacos. • Sarcoma • • a. b. Figure 27. Unclassified sarcoma in a 29-year-old man with cough, transplantation. (a) Coronal T1-weighted (714/12) MR image shows tensity involving the left side of the heart. (b) Photograph of the excis left atrial wall and mitral valve. a. b. Figure 28. Angiosarcoma in a 28-year-old woman who presented with mitral valve obstruction. (a) Axial contrastenhanced chest CT scan (mediastinal window) shows a large nodular, homogeneous, soft-tissue mass that invades the right atrium and encases the heart. (b) Coronal T2-weighted (3,780/57) MR image demonstrates extensive circumferential cardiac involvement by the nodular, heterogeneous, hyperintense tumor, which invades the right atrium and encases the heart. Los más frecuentes. Angiosarcomas: células malignas que Radiologic Features.—The most common raenhancing areas typically corresponding to necrodiographic abnormality in patients with forman canales is cardiomegaly. Other findingscardiac vasculares. sarcoma include sis (91,97,98). MR imaging typically demonstrates cardiac heart failure, pleural effusion, focal cardiac mass, angiosarcomas as large, heterogeneous, invasive pulmonary consolidation, and pericardial effuright atrial masses, frequently with extensive perision (2,7,91,94–96). Because of their location, cardial involvement and hemorrhagic pericardial the radiographic findings of left atrial sarcomas effusion. Signal intensity in these tumors is hetcan mimic those of left atrial myxoma. erogeneous, intermediate on T1-weighted images CT is helpful in the evaluation of cardiac sarwith higher signal intensity on T2-weighted imFigure 26. Angiosarcoma, microscopic features. comas as it demonstrates the broad-based tumor ages (Fig 28). A case report describes the MR High-power photomicrograph (original magnification, 450; H-E stain) demonstrates irregular anastomosing attachment; myocardial, pericardial, and mediasimaging appearance of a polypoid right atrial Clifford-Roberts, W. Primary and secondary well as extension into the greatCardiol.mass with extensive671-682; endothelial cells.and neoplasms of the heart. Am J Volumechannels linedpericardial involvement vascular 80, 1997: by atypical tinal invasion; as Gerenc et al. Primary cardiac andand pulmonary metastases, when present vessels pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No reveals that after intravenous gadolinium admin4:2000:1073-1103. (11,12). Angiosarcomas have been described as istration, the pericardial tumor demonstrated • Predominan en VD. mor at these s myxom Micros ance of tually a sues ha most c (Fig 26 (includ coma) stroma nign m ated sa tural, o Pathol of card majorit masses endoca large, i
    • Tumores Malignos Sarcomas • • • Rabdomiosarcoma: 20%. Sin localización específica. Fibrosarcoma: tumores de “carne blanca”. • • Compuestos de células gigantes fusiformes. Áreas extensas de necrosis y hemorragia. Leiomiosarcoma: células gigantes fusiformes. • • Aurícula derecha. Metastatizan y recurren frecuentemente. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682; Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No 4:2000:1073-1103.
    • Tumores Malignos Sarcomas • Tratamiento • • • • • • Cirugía. Mortalidad secundaria a recurrencia. Supervivencia media: 6 meses a 1 año. Quimioterapia y/o Radioterapia. Trasplante cardiaco. Autotrasplante cardiaco. Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: 671-682; Gerenc et al. Primary cardiac and pericardial neoplasm: Radiologic - Pathologic correlation. AFIP Archives. Vol 20, No 4:2000:1073-1103.
    • qu be ent im Th 60 yea po Tumores Malignos Linfomas • • Proveniente de miocardio. Pronóstico pobre. b. gure 30. Primary cardiac lymphoma in a 75-year-old woman with ogressive dyspnea, superior vena cava syndrome, and atrial fibrillan. (a) Coronal T1-weighted (571/12) MR image shows vena caval vasion (arrow) by a mass. (b) Superior vena cavogram demonstrates e large intraluminal tumor that obstructs the vena cava. Note the coleral blood flow through the azygos and hemiazygos veins. (c) Photoaph of the specimen of the heart obtained at autopsy shows a firm, hite, multinodular right atrial tumor with plaquelike pericardial infiltion (arrow) and obstruction of the superior vena cava ( ). 1096 July-August 2000 RG ■ Volume 20 • Number 4 Figure 29. Lymphoma, microscopic features. Highpower photomicrograph (original magnification, 300; H-E stain) demonstrates lymphoma cells (dark blue round cells) surrounding and infiltrating residual myocytes (arrow). (2,40). Aggressive surgery offers significant palliation of symptoms (caused by valvular and vascular obstruction) and improves survival (89, 92,94). Even after complete tumor excision, however, local recurrence and metastatic disease occur frequently and early, usually within 1 year (2,90,94). Heart transplantation has been performed in some patients with unresectable cardiac sarcoma with satisfactory results (92,95, 100). Chemotherapy and radiation therapy have not proved beneficial for the treatment of affected patients (92). Death in these patients usually results from postoperative complications, cardiopulmonary failure from progressive tumor growth, and metastatic disease (2). adiologic Features.—Chest radiography of tients with primary cardiac lymphoma usually monstrates cardiomegaly, pericardial effusion, d signs of heart failure. Echocardiography typilly demonstrates hypoechoic myocardial masses the right atrium or ventricle with an associated a. b. Primary Cardiac Lymphoma ricardial effusion (102–104,106). At CT, carFigure 30. Primary cardiac lymphoma in a 75-year-old woman with c. Clifford-Roberts, W. Primary and secondary neoplasms ac lymphomas are hypoattenuatingsyndrome,neoplasm:of the heart.-Am J Cardiol. Volume 80, 1997: 671-682; 20, No or iso- and atrial fibrillaprogressive dyspnea, superior vena cava Gerenc et al. Primary cardiac and pericardial Radiologic Pathologic correlation.Clinical Features.—Primary cardiac lymphoAFIP Archives. Vol mas are typically of the non-Hodgkin type. By tion. (a)relative T1-weighted (571/12) MRand dem- vena caval Coronal to the myocardium image shows enuating 4:2000:1073-1103. definition, these tumors involve only the heart or pro car dro the dia ma ear ma pai tha deb du Mi dia Th typ ph lar Bu ph epi the Pa ma ma scr pea Th lym rig sep car alt diu tig
    • Myxo Rhabdomyo Synovial Lipo Schwannoma Lymphoma 8 6 4 2 1 7 (5%) 8 2 4 0 1 1 0 4 0 2 0 6 1 3 0 0 0 0 Breast Mouth & tongue Thyroid gland Uterus Urinary bladder Stomach Colon & rectum Prostate gland Pancreas Nose (interior) Ovary Pharynx Miscellaneous 100 100 100 100 100 100 100 100 100 100 100 100 100 Neoplasias secundarias Totals 144 (100%) 117 (81%) 27 (19%) 11 (8%) *Modified from: Burke A, Virmani R. Atlas of Tumor Pathology. Tumors of the Heart and Great Vessels. Washington, DC: Armed Forces Institute of Pathology 1996:231. • • • • Metástasis. • Clínica: síntomas CV en pacientes ya conocidos con cáncer. • Descartar toxicidad por quimioterapia. TABLE III Metastatic Neoplasms in the Heart at Necropsy— Order of Frequency of Cancers Encountered* Primary Tumor Total Autopsies 8% de todos los cánceres. 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. Lung Breast Lymphoma Leukemia Esophagus Uterus Melanoma Stomach Sarcoma Oral cavity & tongue Colon & rectum Kidney Thyroid gland Larynx Germ cell Urinary bladder Liver & biliary tract Prostate gland Pancreas Ovary Nose (interior) Pharynx Miscellaneous 10% 9% 9% 8% 6% 5% 5% 4% 3% 3% 1% 1% 0 *These percentages were obtained by combining studies by McAllister and Fenoglio and by Mukai et al. (see Table III). Metastases to Heart 1,037 685 392 202 294 451 69 603 159 235 440 114 97 100 21 128 325 171 185 188 32 67 245 180 70 67 66 37 36 32 28 24 22 22 12 9 9 8 8 7 6 6 2 1 1 0 6,240 653 (10%) Vía hematógena o invasión directa. (17%) (10%) (17%) (33%) (13%) (8%) (46%) (5%) (15%) (9%) (5%) (11%) (9%) (9%) (38%) (6%) (2%) (4%) (3%) (1%) (3%) (1%) Melanoma, pulmón, mama, sarcomas, riñón, esófago, hígado, tiroides, leucemia y linfoma. *Modified from: Burke and Virmani (who combined studies of McAllister HA and Fenoglio JJ Jr). Tumors of the Cardiovascular System Atlas of Tumor Pathology. Washington, DC: Armed Forces Institute of Pathology 1978: 111– 119; and Mukai K, Shinkai T, Tominaga K, Shomosato Y. The incidence of secondary tumors of the heart and pericardium: a 10-year study. Jpn N Clin Oncol 1988;18:195–201. FIGURE 1. Diagram showing various locations of cardiac neoplasms involving the heart. LA 5 left atrium; LV 5 left ventricle; RA 5 right atrium; RV 5 right ventricle. in the right atrial cavity (Figure 3), and about 2% in a ventricular cavity.4 –16 On rare occasion, Clifford-Roberts, W. Primary and secondary neoplasms of the heart. Am J Cardiol. Volume 80, 1997: the tumor is present in more than 1 cavity.17 Generally, the neo671-682. plasm when located in the left atrium produces symp-